New Guidelines Address Cushing's Syndrome Treatment

Miriam E Tucker

August 05, 2015

Optimal treatment of Cushing's syndrome (CS) involves direct surgical removal of the causal tumor, while second-line therapy should be individualized, according to new guidelines from the Endocrine Society

"Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline" was published online July 29, 2015 in the Journal of Clinical Endocrinology and Metabolism.

"It's important to individualize the choice of therapy and educate the family about the treatment choices so they can participate in the decision. But in general, the optimal treatment is the one that removes the problem," guideline panel chair Lynnette K Nieman, MD, senior investigator at the Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, told Medscape Medical News.

The new document is the first from the society to address treatment of Cushing's syndrome, which is characterized by an excess of the hormone cortisol in the blood, which is usually caused by a tumor. Previous guidelines addressed diagnosis.

Normalize Cortisol Levels and Then Individualize Treatment

In patients with overt CS, the panel recommends normalizing cortisol levels or action at its receptors to eliminate the signs and symptoms of CS and treating comorbidities associated with hypercortisolism.

Initial resection of the primary lesion(s) underlying the syndrome is the recommended first-line treatment, unless surgery isn't possible or is unlikely to significantly reduce the glucocorticoid excess.

Following surgery, the panel suggests an individualized management approach based on the patient's postoperative serum cortisol values and provides detailed information about glucocorticoid replacement for patients who are hypocortisolemic, including how to taper it. "That's an area where there are several different approaches," Dr Nieman noted.

The document outlines the second-line therapeutic options for patients in whom surgery was not curative or who cannot undergo surgery, including repeat transsphenoidal surgery, radiotherapy, medical therapy, and bilateral adrenalectomy.

"We try to help people see the pros and cons of the different therapies. We're very much oriented toward individualization. For this particular condition, while the number-one treatment is surgery, you can't always do surgery because you can't always find the tumor or it's metastatic, so we want to individualize," Dr Nieman told Medscape Medical News.

Long-Term Follow-Up by Experts Is Key

Cushing's syndrome is a long-lasting condition that can be life-threatening because of its complications, including diabetes, high blood pressure, and depression.

An important section in the guideline, Dr Nieman added, is that which addresses long-term follow-up.

The panel recommends lifelong testing for recurrence and education of patients and families about the clinical features of recurrence. "For most causes but not all, the patient has a chance for recurrence. It's important not to just say you're cured and dismiss them.…It's important to follow up, about annually."

Dr Nieman noted that the seven members of the guideline panel — from the United States, the United Kingdom, and France — are all CS experts.

"CS patients are typically referred to centers of excellence. That's another point we make, than when a patient has CS it makes sense to make treatment decisions in conjunction with someone who has a lot of experience."

The European Society of Endocrinology is a cosponsoring association. Dr Nieman receives compensation for writing chapters on Cushing's syndrome for UpToDate and has received research funding from HRA Pharma. The coauthors report no relevant financial relationships.

J Clin Endocrinol Metab. Published online July 29, 2015. Article


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